Program Operations Manual System (POMS)
TN 53 (08-22)
DI 23022.117 Blastic Plasmacytoid Dendritic Cell Neoplasm
COMPASSIONATE ALLOWANCES INFORMATION
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BLASTIC PLASMACYTOID DENDRITIC CELL NEOPLASM
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ALTERNATE NAMES
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BPDCN; Blastic plasmacytoid dendritic cell neoplasm; CD4+/CD56+ hematodermic neoplasm;
Lymphoblastoid variant of NK-cell lymphoma; Blastic NK-cell lymphoma; Monomorphic
NK-cell lymphoma
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DESCRIPTION
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Blastic plasmacytoid dendritic cell neoplasm (BPDCN) previously known as natural killer (NK) cell leukemia/lymphoma is a rare, clinically
aggressive hematologic malignancy that most commonly manifests as cutaneous lesions
with or without bone marrow involvement and leukemic dissemination.
Common misdiagnoses for BPDCN include non-Hodgkin lymphoma (NHL), acute myeloid leukemia
(AML), leukemia cutis (a nonspecific term used for cutaneous [skin] manifestation
of any type of leukemia), melanoma (a type of skin cancer), and lupus erythematosus
(chronic inflammatory disease that occurs when your body's immune system attacks your
own tissues and organs).
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM
CODING
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Diagnostic testing: Diagnosis of BPDCN is difficult and the condition is often misdiagnosed.
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A history and physical exam;
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Skin biopsy showing infiltration of medium-sized (i.e., immature) blast cells into
the dermis; and
Physical findings: Individuals with BPDCN may present with:
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Skin lesions (nodules, tumors, red or purple papules, bruise-like patches, and/or
ulcers);
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Hepatomegaly (enlarged liver);
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Splenomegaly (enlarged spleen);
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Anemia (decreased red blood cells);
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Thrombocytopenia (abnormally low platelet level); and
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Leukopenia (decreased white blood cells).
ICD-9: 202.9
ICD-10: C86.4
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PROGRESSION
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The skin is the most frequently involved site of disease. However, BPDCN usually progresses
with bone marrow involvement and a decrease in red blood cell, white blood cell and
platelet counts. The lymph nodes and spleen may also be involved. Rashes without symptoms
can also occur.
Due to the high rates of recurrence following initial therapy and the short overall
survival times of individuals with BPDCN, prognosis of the disease is poor.
The average age at diagnosis is 60 to 70 years.
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TREATMENT
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Treatment sometimes includes therapies that are used for AML, acute lymphoblastic
leukemia (ALL), or lymphoma.
The length for which a patient responds to these treatments is usually short. After
a relapse, second remissions with conventional chemotherapy are difficult to achieve.
Allogeneic hematopoietic stem cell transplant (allo-HCT), especially if offered in
first remission, may result in longer remissions. The current recommendation is for
BPDCN patients to be evaluated for an allo-HCT as soon as possible and to begin searching
for a donor.
Other treatment regimens that include intrathecal chemotherapy and hematological stem
cell transplantation in initial treatment regimens and newer non-chemotherapeutic
drug treatments may improve this situation.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for Evaluation:
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Clinical history and examination that describes the diagnostic features of the impairment;
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Skin biopsy showing infiltration of medium-sized (i.e. immature) blast cells into
the dermis; and
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Suggested Listings for Evaluation:
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DETERMINATION
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LISTING
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REMARKS
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Meets |
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Equals |
13.06 A
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested evaluating the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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